The treatment of adult-onset Still's disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature

Clin Exp Rheumatol. 2021 Jan-Feb;39(1):187-195. doi: 10.55563/clinexprheumatol/fsq5vq. Epub 2020 May 12.

Abstract

Adult-onset Still's disease (AOSD) is a rare, inflammatory disease of unknown aetiology, generally affecting young adults and requiring immunosuppressive treatment. In the last few years, bio- logic disease-modifying anti-rheumatic drugs (bDMARDs) have been successfully used in refractory cases, based on the pathogenic role of inflammatory cytokines in AOSD. Amongst bDMARDs, several observations confirmed the clinical usefulness of anakinra, a recombinant human non-glycosylated IL-1 receptor antagonist, in AOSD. At present, the treatment is still largely empirical and due to the possible fallacious aspects of clinical judgement, in this work, we performed a systematic review of literature (SRL) to summarise the evidence regarding the treatment with anakinra in AOSD, analysing rate of complete remission, corticosteroids (CCSs)-sparing effect, long-term retention rate, and safety. After screening titles, abstracts and analysis of full text, 15 manuscripts were analysed: 1 open randomised multicentre trial with two parallel groups and 14 observational single-arm retrospective studies. Collectively, results of the present SRL suggest the effectiveness of anakinra in the treatment of patients with AOSD. Furthermore, patients with AOSD are likely to achieve a good clinical response with anakinra and these outcomes are associated with a largely favourable safety profile. Furthermore, the majority of patients treated with anakinra may achieve a complete remission, also in monotherapy. Finally, the treatment with anakinra is associated with an important CCSs-sparing effect, and, a large percentage of these patients may stop CCSs, thus reducing predictable long-term CCSs side effects without the occurrence of new flares.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Antirheumatic Agents* / adverse effects
  • Humans
  • Interleukin 1 Receptor Antagonist Protein* / adverse effects
  • Interleukin 1 Receptor Antagonist Protein* / therapeutic use
  • Receptors, Interleukin-1
  • Retrospective Studies
  • Still's Disease, Adult-Onset* / diagnosis
  • Still's Disease, Adult-Onset* / drug therapy
  • Young Adult

Substances

  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein
  • Receptors, Interleukin-1